http://www.ijos.org.cn Paramkusam et al. Gangrenosum with Oral Involvement doi: 10.4248/IJOS10032

CASE REPORT

Pyoderma Gangrenosum with Oral Involvement – Case Report and Review of the Literature

Geetha Paramkusam, Venkateswarlu Meduri, Naresh Gangeshetty* Department of Oral Medicine and Radiology, Kaminein Institute of Dental Sciences, Nagonda, India

Abstract a few mm to several cm and have been reported to have been found on the tongue, soft and hard palate, buccal Pyoderma gangrenosum (PG) is a rare, noninfectious neutro- mucosa, and gingiva. Some of these oral lesions have been philic dermatosis. Clinically, it begins with sterile pustules associated with , inflammatory bowel that rapidly progress into painful ulcers of variable depth and disease, and polycythemia rubra vera. A few cases were size with undermined violaceous borders. The diagnosis of reported with biopsy findings, the histological picture being PG is based on the history of an underlying disease, a typical nonspecific, showing ulceration, and necrosis with inflam- clinical presentation, histopathology, and exclusion of other matory cell infiltrate. A peculiar case of pyoderma gan- diseases. The peak incidence occurs between the ages of 20 grenosum with an oral lesion is presented here, and the to 50 years with women being more often affected than men. differential diagnosis is discussed. There have been very few reports of pyoderma gangrenosum with oral mucosal involvement. Oral lesions in previously Keywords pyoderma gangrenosum, oral lesion of pyo- reported cases have included ulcers of varying sizes from derma gangrenosum, diagnosis

Received Jan. 28, 2010; Revision accepted Mar. 23, 2010

Introduction can be seen in patients with ulcerative colitis and crohn’s disease (Bork et al., 1993). Only a few Pyoderma gangrenosum is a rare dermatolo- cases of pyoderma gangrenosum with involvement gical disease. It is primarily a sterile inflammatory of the oral cavity have, however, been reported neutrophilic dermatosis. Its etiology is unknown, (Margoles and Wenger, 1961; Basu and Asquith, but it may be an autoimmune disease. It is 1980; Yusuf and Ead, 1985; Snyder, 1986; Ken- characterized by recurrent cutaneous ulcerations nedy et al., 1987; Yco et al., 1988; Setterfield et with mucopurulent or hemorrhagic exudate. These al., 2001; Hiromi, 2008). very painful ulcers present with undermined bluish borders and surrounding . Skin lesions are mostly ulcerative with central necrosis. Extra- Case Report cutaneous involvement is reported to occur in bone and lungs (Burns et al., 2004; Wollina, 2007). A 42-year-old female patient (Figure 1) reported Pyoderma gangrenosum may occur in the form of to the department of oral medicine and radiology deep ulcers in the oral cavity that sometimes of the Kamineni Institute of Dental Sciences at ulcerate through the tonsillar pillar (Greenberg and Narketpally with symptoms of an on the hard Glick, 2003). Pyoderma gangrenosum-like lesions palate and loosening of the upper front teeth for

Int J Oral Sci, 2(2): 111–116, 2010 - 111 - Pyoderma Gangrenosum with Oral Involvement Paramkusam et al. http://www.ijos.org.cn three months. The patient first noticed a papule on There was grade Ⅲ mobility with 13, grade the hard palate three months prior, this papule then Ⅱ mobility with 12 and 11. Her past medical rupturing, with concomitant exposure of bone. history revealed that she was suffering from The ulcer thus formed extended anteriorly between multiple ulcers of the skin on the lower and upper teeth 12 and 13. extremities, and on the stomach, for the prior three years. These lesions were recurrent (see Figure 3). The lesions were diagnosed as pyoderma gang- renosum by a dermatologist. The patient had been receiving , dapsone, cyclosporine, and metrogyl DG gel.

Figure 1 Extra oral view of patient

On clinical examination: a solitary ulcer was seen on the anterior hard palate, the ulcer being about 4 cm × 2 cm in size, elliptical in shape, with undermined edges. The floor of the ulcer was covered by necrotic bone and pus discharge was noted. The surrounding area of the ulcer was red and inflamed. The ulcer was tender on palpation (Figure 2).

Figure 3 Healed skin lesions on hand and legs

Investigations included complete blood picture, bleeding time, clotting time, serum HBs and HIV detection, liver function tests, random blood sugar, random urine sugar, creatinine level in the urine and complete urine examination, all within normal limits. Maxillary anterior occlusal view (Figure 4) showed a radiolucency in the palate i.r.t. apical region of 11, 12, and 13 teeth. Orthopantamograph (Figure 4), submento-vertex and antero-posterior views of skull were normal. Swab and smear examination were normal. Incisional biopsy from the labial gingiva of upper right lateral incisor and anterior hard palate was performed (Figure 5). Examination of thespecimen from the oral lesion under microscope (Figure 6) showed stratified squamous epithelium, subepithelial connective tissue with a central necrotic area, a collection of Figure 2 The intraoral lesion from labial and palatal , surrounded by dense collections of aspect lymphocytes, plasma cells, and many proli ferating

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Figure 4 Occlusal radiograph revealing the radiolucency in the palate in relation to the 12,13 region and orthopantamograph

tissue with infiltration of and few lymphocytes and eosinophils along with swollen necrosed blood vessels. These features were con- sistent with pyoderma gangrenosum. Differential diagnosis included osteo-myelitis of the maxilla, deep fungal infection like histoplas- mosis, blastomycosis, mucormycosis, and Wegener’s granulomatosis. As the biopsy report was not suggestive of any of other diseases, and pyoderma Figure 5 Surgical specimen of the lesion lesions are expected to show only necrosis and Yellow arrow indicates specimen from gingiva and red arrows inflammatory reaction, it was finally concluded indicate specimen from palate. the patient had an oral mani-festation of pyoderma gangrenosum. capillaries. The histopathological picture was sug- Treatment included extraction of 12, 13 with gestive of a chronic, non-specific suppurative debridement of the lesion. The patient was also inflammatory lesion. The hard tissue section showed advised to use metronidazole ointment thrice daily numerous fragments of bone along with necrotic over the lesion and to use of chlorhexidine mouth debris. Incisional biopsy from the skin lesion rinse thrice daily, as well as 30 mg showed thickening and acanthosis of the daily and dapsone. The patient also developed a with mild spongiosis. There was elongation and fresh lesion on the left retromolar area after two uniform broadening of rete ridges with focal weeks, while the lesion on the palate was partially blurring of the basement membrane. The papillary healed. Both of the lesions were completely healed dermis showed swelling and necrosis of connective after 6 weeks (Figures 7 and 8).

Figure 6 Examination of specimen from the gingival lesion under microscope At low power (× 10), showing the necrotic area surrounded by inflammatory cells and at high power (× 45), showing the inflammatory cells.

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Figure 7 Palatal lesion two and six weeks after initiation of treatment

Figure 8 Lesion in right buccal mucosa which developed two weeks after the initial visit and which healed after four weeks of treatment

purulent exudate at the ulcer base. Lesions may be Discussion solitary or multiple, commonly found on the lower legs, followed by the thighs, buttocks, chest, head, Pyoderma gangrenosum is a rare, neutrophilic and neck. Generally, lesions are less than 10 cm in dermatosis commonly associated with underlying size, but can be very large. Lesions tend to endure, systemic disease. Pyoderma gangre-nosum was lasting months to years and heal with an atrophic first described and named by Brunsting, Goecker- cribri-form . Associated symptoms include fever, man and O'Leary in 1930 (Burns et al., 2004). malaise, myalgia, and arthralgia. Extracutaneous Approximately 50% of patients have an associated involvement is reported in bone and lungs with systemic disease, commonly inflammatory bowel neutrophilic infiltrate. The disease may have a disease (IBD), ulcerative colitis, arthritis, hema- variety of clinical presentations: ulcerative, pustular, tological and lymphoreticular malignancies, or bullous or atypical, vegetative or superficial granulo- diseases known to be associated with significant matosis (Burns et al., 2004; Wollina, 2007). immunocompromise, while in another 40%–50% Oral involvement in pyoderma gangrenosum is patients, no underlying disease is found. The uncommon. The oral lesions may appear as disease is recurrent in approximately 30% of irregular shaped ulcers 15 mm to 20 mm in patients (Setterfield et al., 2001; Burns et al., diameter with rolled out margins and a grayish 2004). It predominately occurs in adults between colored base. The lesions are painful (Yusuf and the ages of 25–54 years; rarely in children. The Ead, 1985). Pyostomatitis vegetans is considered classic presentation begins with small tender to be the oral counterpart of pyoderma gang- papules, pustules or nodules which breakdown renosum (Bork et al., 1993). This is another rare centrally and rapidly progress to a painful ulcer condition which can occur in association with with characteristic violaceous undermined edges. ulcerative colitis and is characterized by active There may be granulation tissue, necrosis or exacerbations which often coincide with those of

- 114 - Int J Oral Sci, 2(2): 111–116, 2010 http://www.ijos.org.cn Paramkusam et al. Pyoderma Gangrenosum with Oral Involvement the colitis. The lips and cheeks are diffusely swollen For extensive disease, systemic corticosteroids and with deep fissure-like ulcerations separating papillary cyclosporins are used. For more severe disease, projections of the mucous membrane. The patient oral corticosteroids, sulphones and other anti- is often pyrexial with generalized lymphadeno- microbial agents like dapsone, clofazimine, mino- pathy (Yusuf and Ead, 1985). cycline are used. Refractory cases may need Previously reported cases of prominent oral pulsed intravenous therapy. Local mucosal involvement in patients with pyoderma therapy of the lesions includes pain relief, pre- gangrenosum have indicated that the sites of vention of secondary bacterial infections and involvement are the tongue, palate, lips, buccal dressings (Burns et al., 2004; Wollina, 2007). mucosa, gingiva and tonsillar fauces (Margoles The patient we have described had classical and Wenger, 1961; Basu and Asquith, 1980; Yusuf lesions of pyoderma gangrenosum of the legs, and Ead, 1985; Snyder 1986; Kennedy et al., 1987; wrist and abdomen which were healed. There was Yco et al., 1988; Setterfield et al., 2001; Hiromi, no evidence of inflammatory bowel disease in this 2008). In five of the previously reported cases, PG patient or any of the other described disease was associated with inflammatory bowel disease, associations. The lesion on her palate was usually ulcerative colitis (UC). consistent with previous reports of a chronic The histopathology of the skin lesions is often inflammatory lesion with neutrophils. Its occur- variable and nonspecific, but can be useful in rence in association with pyoderma gangrenosum excluding other possible etiologies. Findings include: elsewhere and its very rapid resolution with central necrosis and ulceration of the epidermis systemic steroids suggests that it was also a and dermis surrounded by an intense, acute manifestation of this disease. inflammatory cell infiltrate, with a more peripheral mixed-to-chronic inflammatory cell infiltrate (Burns References et al., 2004; Wollina, 2007). The histopathology of the oral lesions in previously reported cases shows Basu M, Asquith P (1980). Oral manifestations of inflam- necrosis, ulceration with an overlying fibrino- matory bowel disease. Clin Gastroenterol, 9(2): 307– purulent membrane; heavy infiltration of the 321. lamina propria with chronic inflammatory cells Bork K, Hoede N, Korting GW, Burgdorf WHC, Young predominately neutrophils and perivascular hyali- SK (1993). Diseases of the oral mucosa and the lips. nization and fibrin deposition (Margoles and 3rd ed. Philadelphia: Saunders, p.46. Wenger, 1961; Yco et al., 1988; Setterfield et al., Greenberg MS, Glick M (2003). Burket’s oral medicine:

2001). diagnosis and treatment. 10th ed. Hamilton: BC Decker, As there are no diagnostic features on biopsy, it p.397. is of little diagnostic value. The aim of the biopsy Hiromi T (2008). Case of pyoderma gangrenosum showing is to rule out diagnoses that mimic pyoderma oral and genital ulcers, misdiagnosed as Behcet’s gangrenosum. No specific laboratory test available. disease at first medical examination. J Dermatol, 35(5): Laboratory investigations are done to identify 289–292. associated diseases and to rule out diagnoses that Kennedy KS, Prendergast ML, Sooy CD (1987). Pyoderma mimic pyoderma gangrenosum (Burns et al., 2004; gangrenosum of the oral cavity, nose and larynx. Wollina, 2007). Therefore, the diagnosis is pri- Otolaryngol Head Neck Surg, 97(5): 487–490. marily clinical and often a diagnosis of exclusion. Margoles JS, Wenger J (1961). Stomal ulceration associated Features of the ulcer on clinical examination with pyoderma gangrenosum and ulcerative colitis. include tenderness, necrosis, irregular violaceous Report of two cases. Gastroenterology, 41: 594–598. border, with undermined, rolled edges. Setterfield JF, Shirlaw PJ, Challacombe SJ, Black MM Treatment depends on the disease and the (2001). Pyoderma gangrenosum associated with severe presence of associated diseased. Systemic therapy oropharyngeal involvement and IgA paraproteinaemia. for early or mild lesions included topical therapy Br J Dermatol, 144(2): 393–396. with hydrophilic occlusive dressings, antimicrobial Snyder RA(1986). Pyoderma gangrenosum involving the agents or topical or intralesional corticosteroids. head and neck. Arch Dermatology, 122(3): 295–302.

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Burns T, Breathnach S, Cox N, Griffiths C (2004). Rook’s Pyoderma gangrenosum involving the head and neck. textbook of dermatology. Vol.3, 7th ed. Massachusetts: Laryngology, 98(7): 765–768. Blackwell publishing, p.36–39. Yusuf H, Ead RD (1985). Pyoderma Gangrenosum with Wollina U (2007). Pyoderma gangrenosum – a review. Involvement of the Tongue. Br J Oral Maxillofac Surg, Orphanet J Rare Dis, 2:19. 23(4): 241–250. Yco MS, Warnock GR, Cruickshank JC, Burnett JR (1988).

*Corresponding author: Naresh Gangeshetty Address: Department of Oral Medicine and Radiology, Kaminein Institute of Dental Sciences, Sreepuram, Narketpally, Nagonda, Andhra Pradesh, India Tel: 9866063380 E-mail: [email protected]

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